Methods are being developed for rapid, high-resolution analytical and preparative separations and analyses of glycosaminoglycans. These methods are based upon the partition between immiscible phases of complexes formed between the glycosaminoglycan polyion and various organic cations. Currently the complexes under investigation are those formed with quaternary ammonium salts which can be partitioned between aqueous and butanolic phases. The partition properties of various mucopolysaccharide preparations are being systematically investigated as functions of solution conditions to determine criteria for the identification of unknown mixtures. The technique of countercurrent chromatography, which is a technique for making continuous flow separations by partition without the necessity of using an inert support, is being applied to the problem of obtaining high-resolution separations. After the development of appropriate methods for the separation and determination of glycosaminoglycans in body fluids such as urine and amniotic fluid, the qualitative and quantitative glycosaminoglycan composition of urine and amniotic fluid will be determined as these relate to normal and abnormal metabolism. Specifically, the urinary excretion patterns will be determined for normal individuals, individuals affected with the genetic mucopolysaccharidoses, and individuals affected with other disorders producing abnormalities in glycosaminoglycan composition. These studies will be used to better define the normal state, as well as providing information which can be used to derive improved diagnostic criteria from glycosaminoglycan analyses. BIBLIOGRAPHIC REFERENCE: R.E. Hurst, R.C. Cezayirli, and A.E. Lorincz. The Nature of the Glycosaminoglycanuria (Mucopolysacchariduria) in Brachycephalic "Snorter" Dwarf Cattle. Journal of Comparative Pathology, 85, 481-487 (1975).